Presentation
[…] characteristic radiologic features of uremic leontiasis ossea. 6 Case Report We present case report of two patients (12-year-old girl and 29- year-old male) presenting with jaw masses. [slideplayer.com]
This study presents a report on a patient with advanced hyperparathyroidism with skeletal changes compatible with leontiasis ossea. [journalrip.com]
Entities such as cherubism usually present at 2-3 years of age and spontaneously resolve after puberty. 8 Paget’s disease typically presents later than the current patient’s age and has a male predilection. [appliedradiology.com]
The present case emphasizes the need to evaluate pathology specimens in the appropriate clinical context. [ajnr.org]
Entire Body System
- Developmental Disorder
Although FD is linked to an activating mutation in the gene that encodes the subunit of stimulatory G protein (Gs) located at 20q13.2–13.3, it is a non-heritable congenital developmental disorder. [doi.org]
- Dentist
Spec Care Dentist. 2003;1:28-34. Yang Q et al. Skeletal Lesions in primary hyperparathyroidism. Am J Med Sci. 2015;349(4):321-327. Cure JK, Vattoth S, Shah R. Radiopaque jaw lesions: an approach to the differential diagnosis. [appliedradiology.com]
Respiratoric
Jaw & Teeth
- Mouth Breathing
Physical examinations revealed: toggled speech (nasal speech), mouth breathing and facial change, saddle nose, nares widening, increased interdental space and mandibular enlargement. [journalrip.com]
Although he had facial deformities since early childhood, he professed only to cosmetic embarassment, nasal blockage, mouth breathing, mild snoring and progressing bowing of his shins. There were no other affected family members. [doi.org]
- Widely Spaced Teeth
Physical exam revealed marked non-tender maxillary and mandibular hypertrophy with nasal flattening, wide-spaced teeth and severe palatal changes. [file.scirp.org]
Eyes
- Lagophthalmos
There was a mild degree of lagophthalmos on the right side. The lids were swollen, with prominent veins in the upper lids. The inner halves of the lids were reddened. [ijo.in]
Musculoskeletal
- Osteopenia
Radiologically, the maxillofacial changes of renal osteodystrophy have previously been broken down into three separate patterns. 3 Osteitis fibrosa is a constellation of osteolytic lesions, cortical thinning, and coarsened trabeculation. 4,5 Microlunar osteopenia [appliedradiology.com]
Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior [file.scirp.org]
- Bony Swelling
The left half of the face was more prominent than the right half on account of swelling of the zygomatic and maxillary region [Figure - 1]. There was a bony swelling on the left frontal region which according to him was present since birth. [ijo.in]
- Hand Muscle Weakness
He was discharged home on the 10 th day with residual distal hand muscle weakness and grade 4 muscle power in his legs. [doi.org]
- Saber Shin
He had broad forearms, sausage like fingers and bowing of both legs with saber shins (Figure 2 ). The nasal bridge was flat and elevated due to a soft tissue thickening (Figure 1 & 2 ). No café au lait spots were observed. [doi.org]
- Joint Subluxation
subluxations or dislocations and compressive symptoms [ 1 ]. [doi.org]
Neurologic
- Dysarthria
A 37-year-old female with end-stage renal disease from lupus disease on regular hemodialysis complaining of progressive facial deformity for two years causing dyspnea, dental malocclusion and dysarthria with the final diagnosis of uremic leontiasis ossea [file.scirp.org]
Facial involvement is rarer and often presents clinically with progressive swelling—sometimes painful due to nerve encroachment and tissue ulceration—leading to dysphagia, dysarthria, and airway compromise. 1,2 The term “leontiasis ossea” has been generalized [appliedradiology.com]
Case Report A 37-year-old man with a history of end stage renal disease who was on hemodialysis for 5 years presented with an expanding palatal lesion of 6-months’ duration, which caused dysarthria and oral dysphagia. [ajnr.org]
- Nasal Speech
Physical examinations revealed: toggled speech (nasal speech), mouth breathing and facial change, saddle nose, nares widening, increased interdental space and mandibular enlargement. [journalrip.com]
Workup
X-Ray
- Rugger-Jersey Spine
Findings in secondary (and tertiary) hyperparathyroidism are numerous: sub periosteal bone resorption, osteopenia, osteosclerosis e.g. rugger-jersey spine, soft tissue calcification, generalized increased uptake on Tc-99m pertechnetate bone scan, superior [file.scirp.org]
Hypertrophy
- Ventricular Hypertrophy
Electro-cardiogram : The conclusion from the examination was one of left ventricular hypertrophy. X-ray of skull: A.P. and Lateral views showed diffuse hyperostosis of bone with loss of differentiation between outer and inner tables [Figure - 2]. [ijo.in]
Treatment
It is possible that at this stage of the disease, the patient has tertiary hyperparathyroidism when treatment is rarely successful7. [ncbi.nlm.nih.gov]
A medical practitioner should always be consulted for diagnosis and treatment of all medical conditions. [hospitalhelp.co.uk]
Review the unique radiologic findings, differential diagnosis, treatment and follow up in uremic leontiasis ossea. [posterng.netkey.at]
Prognosis
More often, most lesions can be managed through staged procedures with overall very favorable outcomes and good long term prognosis. [skullbaseinstitute.com]
Treatment usually entails parathyroidectomy with monitoring of serum calcium, phosphate, and vitamin D levels while on appropriate replacement (calcium/vitamin D) or binding (phosphate) pharmacotherapy. 4 The prognosis is usually favorable. [appliedradiology.com]
Etiology
Clinical-Medical Image: Paget disease of the bone is a chronic disorder of unknown etiology that typically results in enlarged, deformed bones due to abnormal and excessive bone remodeling. [imagejournals.org]
Indian J Ophthalmol 1969;17:166-70 Introduction The term leontiasis ossea was first coined by Virchow [6] in 1896 to describe the leonine appearance of the facial deformity in this rare condition of unknown etiology. [ijo.in]
However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5]. After parathyroidectomy, facial changes can be stabilized or improve mildly. [file.scirp.org]
Pathophysiology
Pathophysiological origin of the disease is not clearly understood. However, nutritional and secondary hyperparathyroidism due to uremia has been suggested as major etiologies for the disease [5]. [file.scirp.org]
Prevention
Hence, early recognition and treatment of leontiasis ossea can prevent its complications. Please cite this paper as: Raffiei Jelodar H, Yarmohamadi M, Baghai Wadji M, Babaei MR. Leontiasis ossea; a rare presentation of secondary hyperparathyroidism. [journalrip.com]
Early recognition andconfident diagnosisof ULO is essential to prevent progression to facial disfigurement andavoid invasive procedures. [posterng.netkey.at]
During hemodialysis patients often develop numerous comorbid conditions that may interfere with the existing disease and prevent further treatment, as was the reported case. [ncbi.nlm.nih.gov]
Ultimately, early recognition of incipient uremic leontiasis ossea clinically and on imaging is essential to prevent progression to severe disfigurement that can result from prolonged untreated secondary hyperparathyroidism [12]. [file.scirp.org]
Hence, CKD‐MBD patients should be educated about their illness, early prevention, multidisciplinary early diagnosis, and treatment plans, thereby improving their quality of life and long‐term survival.24 We hope this case report will highlight the awareness [asbmr.onlinelibrary.wiley.com]